Squamous cell liver cancer (SCLC) arising from an epidermoid intestinal cyst is rare. Only 65 cases of this cyst have been reported since 1850, with 2 reported cases of squamous cell cancer. We describe here the case of a 21-year-old man who complained of mild pain, a feeling of fullness in the right upper quadrant of the abdomen, and fever and weight loss, who developed SCLC arising from an epidermoid intestinal cyst. The clinical presentation, management, and pathological findings are discussed.